Ebstein’s Anomaly – Mayo Clinic

Cardiac surgeon Joseph Dearani, M.D., discusses Ebstein’s anomaly, related defects, common symptoms, and treatment. Visit http://www.mayoclinic.org/diseases-co... for more information on Ebstein’s anomaly, symptoms, diagnosis, and treatment options at Mayo Clinic.

Ebstein’s anomaly is a rare congenital heart defect where the tricuspid valve sits lower than normal in the right ventricle. Ebstein’s anomaly can be highly symptomatic at birth requiring urgent surgery; however, in some patients, it may go undetected for many years. The most common presentation in children is cyanosis, however, other conditions such as holes in the heart, arrhythmias, and Wolff-Parkinson-White syndrome may also be associated with Ebstein’s anomaly.

Treatment for Ebstein’s anomaly requires surgical intervention. The goal of surgery is to repair and preserve the native tricuspid valves, and the survival rate is excellent. The vast majority of Ebstein’s anomaly patients will need surgery for symptomatic tricuspid regurgitation, right-sided heart failure, cyanosis or paradoxical embolism.